Autism spectrum disorder 101: A primer for pediatric otolaryngologists.

Based on a comprehensive review of the literature as well as personal experiences at a tertiary children's hospital, we present an encompassing guide of the management of children with autism spectrum disorder (ASD) in the otolaryngology practice. ASD is a disorder involving persistent deficits in social communication and interaction across multiple contexts with restricted and repetitive patterns of behavior, which presents early in age and causes significant impairment in function. With the increasing prevalence of autism, the otolaryngologist's understanding of the disease and how it affects the management of patients is of paramount importance in order to provide a safe medical and surgical environment. Special considerations in the outpatient visit and peri-operative setting in the management of ASD patients can enhance the patient-provider relationship and improve the quality of care delivered. We discuss several strategies, such as utilization of communication devices and use of pre-medication prior to surgery, to facilitate the clinical experience.

Efficacy of powered intracapsular tonsillectomy and adenoidectomy.

OBJECTIVES/HYPOTHESIS: We sought to determine the effectiveness of powered intracapsular tonsillectomy and adenoidectomy (PITA) in the treatment of children with moderately severe obstructive sleep apnea and to measure changes in quality of life that occur with such treatment. STUDY DESIGN: Prospective, nonrandomized clinical trial in an academic pediatric otolaryngology practice. METHODS: Convenience sample of children ages 3 to 12 years diagnosed with obstructive sleep apnea of moderate severity, defined as an apnea-hypopnea index (AHI) between 5 and 20 on polysomnography. Children with recurrent streptococcal pharyngitis, chromosomal abnormalities, craniofacial abnormalities, neuromotor disease, sickle cell disease, obesity, or coagulopathy were excluded. PITA was performed by using the microdebrider. Polysomnography was performed before surgery and repeated 4 to 8 weeks after surgery. The Obstructive Sleep Apnea (OSA)-18 questionnaire was completed at surgery and at the time of postoperative polysomnography to assess quality of life changes. The main outcome measure was cure of obstructive sleep apnea, as defined by a postoperative AHI of 1 or less for complete cure and less than 5 for partial cure. Improvements in quality of life were assessed by changes in the OSA-18 questionnaire. RESULTS: Nineteen children underwent PITA for moderate obstructive sleep apnea syndrome (OSAS), and 14 completed postoperative polysomnography. All 14 subjects who completed the study achieved at least partial cure. Thirteen of 14 (93%) subjects had a complete cure of OSAS after PITA. The median preoperative AHI was 7.9, and the median AHI after surgery was 0.1. The mean number of arousals per hour before surgery was 9.5, and this was reduced to a mean of 5.6 after surgery. Quality of life measures on OSA-18 also improved, with large improvements in total quality of life scores and in all five domains seen after surgery. CONCLUSIONS: PITA cures otherwise healthy children with obstructive sleep apnea of moderate severity, at least in the short-term, as documented by postoperative polysomnography. Improvements in quality of life measures, as documented by changes in OSA-18, were seen in all children as well.

Ion channel proteins in mouse and human vestibular tissue.

BACKGROUND AND OBJECTIVE: Electrical activity in hair cells and neurons of the inner ear is necessary for the transduction and modulation of stimuli that impinge on the cochlea and vestibular endorgans of the inner ear. The underlying basis of this activity is pore-forming proteins in the membrane of excitable cells that allow the influx and efflux of various ions, including Na + , Ca 2+ , and K + , among others. These channels are critical to both electrical activity as well as the development of excitable cells because they may initiate long-term signals that are important in the maintenance and survival of these cells. We investigated the expression of several Shaker potassium ion channel proteins and an accessory beta subunit in the vestibular endorgans of mouse and human. METHODS: Vestibular tissue consisting of cristae ampullares was harvested from adult and neonatal mice as well as from human subjects undergoing vestibular surgery. Western blot analysis and immunoprecipitation were used to identify the presence or absence, in mouse, of alpha subunits Kv1.2, Kv1.4, and Kv1.5 and of beta subunit Kvbeta1.1 in mouse. Coimmunoprecipitation was used to identify interactions between alpha and beta subunits. Immunohistochemistry was used to localize Kv1.2 in mouse and human tissues. RESULTS: The presence of Kvalpha1.2 and Kvbeta1.1 was confirmed in adult mouse crista ampullaris by Western blotting. Coimmunoprecipitation experiments showed that Kv1.2 and Kvbeta1.1 interact in these tissues. Immunostaining localized Kv1.2 to regions within and extraneous to the sensory epithelium of mouse and human cristae ampullares. In comparison, Kv1.4 and Kv1.5 were not found in the crista ampullaris. CONCLUSIONS: We describe the presence, location, and interaction of various potassium ion channel alpha subunits and a beta subunit. These data are initial descriptions of potassium ion channels in the mammalian vestibular system and begin to provide an understanding of the protein subunits that form ion channels of the mammalian inner ear. In addition, our data show that there are interactions that occur that may regulate the biophysical properties of these channels, thereby contributing to the diversity of channel function. This knowledge is critical to understanding the genes that encode these channels and finding cures for pathologies of hearing and balance. SIGNIFICANCE: We detail initial characteristics of potassium ion channel proteins including alpha subunits Kv1.2, Kv1.4, and Kv1.5 and beta subunit Kvbeta1.1 in mammalian vestibular tissue. This knowledge is critical to understanding the processing of vestibular stimuli and the regulation of endolymphatic function. Mutations of ion channels can cause neurological pathologies including auditory and vestibular disorders in humans.

Laryngotracheal injury after percutaneous dilational tracheostomy in cadaver specimens.

OBJECTIVE: To evaluate the stoma and surrounding insertion site for common laryngotracheal injury patterns after percutaneous dilational tracheostomy that may contribute to clinically significant tracheal stenosis. STUDY DESIGN: Preliminary prospective cadaver study. METHODS: A preliminary prospective cadaver study was performed in which percutaneous dilational tracheostomy (Ciaglia Blue Rhino kit) was placed in six fixed cadaveric specimens. Laryngotracheal segments were harvested. Two independent evaluators graded cadaver features and characteristic injuries in laryngotracheal specimens the tracheal site of stoma placement was identified. Cadaver features evaluated included gender, obesity, and ease of landmark identification. Mucosal and cartilage injuries were evaluated on a scale of 0 to 4, with 4 representing severe comminuted injuries beyond the intended stoma site. RESULTS: Accurate prediction of tracheal placement was achieved in only three (50%) of the specimens. In the remaining cadavers, one stoma site was placed higher than intended (16%) and two sites were lower than intended (33%). Mucosal injury was most severe at the anterior internal surface of the trachea with all specimens sustaining tears beyond one tracheal ring (mean mucosal injury score, 3.5). Cartilaginous injury was severe in five of six specimens (83%) that sustained multiple comminuted injuries to two or more adjacent rings (mean cartilage injury score, 3.34). Cricoid comminutions and a posterior membranous tracheal wall injury were each found in one specimen. CONCLUSIONS: Laryngotracheal injuries found after percutaneous dilational tracheostomy in the study indicated that severe damage to mucosa and cartilage surrounding the intended stoma site occurs at the time of placement. These injuries may contribute to clinically significant tracheal stenosis preventing decannulation in patients undergoing percutaneous dilational tracheostomy. Two case studies that support this hypothesis are also presented.